Barreiro-Capurro A, Mascaró-Galy J M, Iranzo P
Servicio de Dermatología, Hospital Clínic, Universitat de Barcelona, Barcelona, España.
Actas Dermosifiliogr. 2013 Dec;104(10):904-14. doi: 10.1016/j.ad.2013.05.005. Epub 2013 Jul 26.
Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease caused by autoantibodies to type VII collagen. The clinical presentation is variable, with skin and mucosal lesions that can cause significant dysfunction. Different treatment options exist, but the results are often unsatisfactory.
To review all the cases of epidermolysis bullosa acquisita (EBA) diagnosed at our hospital over a 26-year period.
We performed a retrospective review of the clinical, histologic, and immunologic features of EBA in 9 patients.
Mean age at presentation was 37 years and 66.67% of the patients were women. EBA occurred in association with malignant tumors, inflammatory bowel disease, and autoimmune disorders. The most common variant was inflammatory EBA (6 of the 9 cases). In all 9 patients, histology revealed a subepidermal blister and direct immunofluorescence showed linear deposits of immunoglobulin G and C3 in the basement membrane zone. Indirect immunofluorescence performed on salt-split skin substrate was positive in 6 patients and showed a dermal pattern in all cases. Five patients were tested for autoantibodies to type VII collagen using enzyme-linked immunosorbent assay, with positive results in 2 cases. Immunoblotting using recombinant noncollagenous domains (NC1) of type VII collagen was positive in all 6 cases in which it was performed. Response to treatment was variable.
EBA is a rare disease with a variable clinical presentation that can be confused with that of other subepidermal blistering diseases. Correct diagnosis requires a high level of clinical suspicion and the use of all available diagnostic tests. Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications.
获得性大疱性表皮松解症（EBA）是一种自身免疫性表皮下大疱性疾病，由针对VII型胶原蛋白的自身抗体引起。临床表现多样，皮肤和黏膜病变可导致严重功能障碍。虽然有不同的治疗选择，但结果往往不尽人意。
回顾我院26年间诊断的所有获得性大疱性表皮松解症（EBA）病例。
我们对9例EBA患者的临床、组织学和免疫学特征进行了回顾性研究。
患者就诊时的平均年龄为37岁，66.67%为女性。EBA与恶性肿瘤、炎症性肠病和自身免疫性疾病相关。最常见的类型是炎症性EBA（9例中的6例）。所有9例患者的组织学检查均显示表皮下大疱，直接免疫荧光显示基底膜区有免疫球蛋白G和C3的线性沉积。对盐裂皮肤底物进行的间接免疫荧光在6例患者中呈阳性，且所有病例均显示真皮型。5例患者使用酶联免疫吸附测定法检测了针对VII型胶原蛋白的自身抗体，2例结果为阳性。使用VII型胶原蛋白的重组非胶原结构域（NC1）进行免疫印迹，在进行检测的所有6例中均为阳性。治疗反应各不相同。
EBA是一种罕见疾病，临床表现多样，可能与其他表皮下大疱性疾病混淆。正确诊断需要高度的临床怀疑并使用所有可用的诊断测试。对皮肤和黏膜受累情况进行全面评估并及时开始适当治疗，将确保发现并预防功能障碍和治疗相关并发症。
